Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome

Pumin Zhang; Nanette J. Liégeois; Calvin Wong; Milton Finegold; Harry Hou; Janet C. Thompson; Adam Silverman; J. Wade Harper; Ronald A. DePinho; Stephen J. Elledge

doi:10.1038/387151a0

Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome

Pumin Zhang, Nanette J. Liégeois, Calvin Wong, Milton Finegold, Harry Hou, Janet C. Thompson, Adam Silverman, J. Wade Harper, Ronald A. DePinho, Stephen J. Elledge

Cell Biology

Research output: Contribution to journal › Article › peer-review

665 Scopus citations

Abstract

Mice lacking the imprinted Cdk inhibitor p57(KIP2) have altered cell proliferation and differentiation, leading to abdominal muscle defects; cleft palate; endochondral bone ossification defects with incomplete differentiation of hypertrophic chondrocytes; renal modullary dysplasia; adrenal cortical hyperplasia and cytomegaly; and lens cell hyperproliferation and apoptosis. Many of these phenotypes are also seen in patients with Beckwith-Wiedemann syndrome, a pleiotropic hereditary disorder characterized by overgrowth and predisposition to cancer, suggesting that loss of p57(KIP2) expression may play a role in the condition.

Original language	English (US)
Pages (from-to)	151-158
Number of pages	8
Journal	Nature
Volume	387
Issue number	6629
DOIs	https://doi.org/10.1038/387151a0
State	Published - May 8 1997

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title = "Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome",

abstract = "Mice lacking the imprinted Cdk inhibitor p57(KIP2) have altered cell proliferation and differentiation, leading to abdominal muscle defects; cleft palate; endochondral bone ossification defects with incomplete differentiation of hypertrophic chondrocytes; renal modullary dysplasia; adrenal cortical hyperplasia and cytomegaly; and lens cell hyperproliferation and apoptosis. Many of these phenotypes are also seen in patients with Beckwith-Wiedemann syndrome, a pleiotropic hereditary disorder characterized by overgrowth and predisposition to cancer, suggesting that loss of p57(KIP2) expression may play a role in the condition.",

author = "Pumin Zhang and Li{\'e}geois, {Nanette J.} and Calvin Wong and Milton Finegold and Harry Hou and Thompson, {Janet C.} and Adam Silverman and Harper, {J. Wade} and DePinho, {Ronald A.} and Elledge, {Stephen J.}",

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doi = "10.1038/387151a0",

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T1 - Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome

AU - Zhang, Pumin

AU - Liégeois, Nanette J.

AU - Wong, Calvin

AU - Finegold, Milton

AU - Hou, Harry

AU - Thompson, Janet C.

AU - Silverman, Adam

AU - Harper, J. Wade

AU - DePinho, Ronald A.

AU - Elledge, Stephen J.

PY - 1997/5/8

Y1 - 1997/5/8

N2 - Mice lacking the imprinted Cdk inhibitor p57(KIP2) have altered cell proliferation and differentiation, leading to abdominal muscle defects; cleft palate; endochondral bone ossification defects with incomplete differentiation of hypertrophic chondrocytes; renal modullary dysplasia; adrenal cortical hyperplasia and cytomegaly; and lens cell hyperproliferation and apoptosis. Many of these phenotypes are also seen in patients with Beckwith-Wiedemann syndrome, a pleiotropic hereditary disorder characterized by overgrowth and predisposition to cancer, suggesting that loss of p57(KIP2) expression may play a role in the condition.

AB - Mice lacking the imprinted Cdk inhibitor p57(KIP2) have altered cell proliferation and differentiation, leading to abdominal muscle defects; cleft palate; endochondral bone ossification defects with incomplete differentiation of hypertrophic chondrocytes; renal modullary dysplasia; adrenal cortical hyperplasia and cytomegaly; and lens cell hyperproliferation and apoptosis. Many of these phenotypes are also seen in patients with Beckwith-Wiedemann syndrome, a pleiotropic hereditary disorder characterized by overgrowth and predisposition to cancer, suggesting that loss of p57(KIP2) expression may play a role in the condition.

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Altered cell differentiation and proliferation in mice lacking p57(KIP2) indicates a role in Beckwith-Wiedemann syndrome

Abstract

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